Diagnosis of hepatic glycogenosis in poorly controlled type 1 diabetes mellitus  

作　　者：Stefania Giordano Antonio Martocchia Lavinia Toussan Manuela Stefanelli Francesca Pastore Antonio Devito Marcello G Risicato Luigi Ruco Paolo Falaschi 

机构地区：[1]Geriatric Unit,"Sapienza" University of Rome,Faculty of Medicine and Psychology,S. Andrea Hospital

出　　处：《World Journal of Diabetes》2014年第6期882-888,共7页世界糖尿病杂志（英文版）（电子版）

摘　　要：Hepatic glycogenosis(HG) in type 1 diabetes is a underrecognized complication. Mauriac firstly described the syndrome characterized by hepatomegaly with altered liver enzymes, growth impairment, delay puberty and Cushingoid features, during childhood. HG in adulthood is characterized by the liver disorder(with circulating aminotransferase increase) in the presence of poor glycemic control(elevation of glycated hemoglobin, Hb A1 c levels). The advances in the comprehension of the metabolic pathways driving to the hepatic glycogen deposition point out the role of glucose transporters and insulin mediated activations of glucokinase and glycogen synthase, with inhibition of glucose-6-phosphatase. The differential diagnosis of HG consists in the exclusion of causes of liver damage(infectious, metabolic, obstructive and autoimmune disease). The imaging study(ultrasonography and/or radiological examinations) gives information about the liver alterations(hepatomegaly), but the diagnosis needs to be confirmed by the liver biopsy. The main treatment of HG is the amelioration of glycemic control that is usu-ally accompanied by the reversal of the liver disorder. In selected cases, more aggressive treatment options(transplantation) have been successfully reported.Hepatic glycogenosis(HG) in type 1 diabetes is a underrecognized complication. Mauriac firstly described the syndrome characterized by hepatomegaly with altered liver enzymes, growth impairment, delay puberty and Cushingoid features, during childhood. HG in adulthood is characterized by the liver disorder(with circulating aminotransferase increase) in the presence of poor glycemic control(elevation of glycated hemoglobin, Hb A1 c levels). The advances in the comprehension of the metabolic pathways driving to the hepatic glycogen deposition point out the role of glucose transporters and insulin mediated activations of glucokinase and glycogen synthase, with inhibition of glucose-6-phosphatase. The differential diagnosis of HG consists in the exclusion of causes of liver damage(infectious, metabolic, obstructive and autoimmune disease). The imaging study(ultrasonography and/or radiological examinations) gives information about the liver alterations(hepatomegaly), but the diagnosis needs to be confirmed by the liver biopsy. The main treatment of HG is the amelioration of glycemic control that is usu-ally accompanied by the reversal of the liver disorder. In selected cases, more aggressive treatment options(transplantation) have been successfully reported.

关 键 词：Hepatic glycogenosis Type 1 diabetes mellitus HEPATOMEGALY GLYCOGEN Glucose transporters Insulin GLUCOKINASE Glycogen synthase GLUCOSE-6-PHOSPHATASE 

分 类 号：R587.1[医药卫生—内分泌]